ABSTRACT
IMPORTANCE: Linear IgA/IgG bullous dermatosis (LAGBD) is a relatively rare autoimmune bullous disease characterized by both IgA and IgG antibodies to epidermal basement membrane zone. The heterogeneity and pathogenesis of the LAGBD autoantigens have not been fully elucidated. OBSERVATIONS: We report 3 Japanese cases of LAGBD (ages 81, 88, and 64 years; 1 woman and 2 men). The patients showed bullous and erosive lesions on the trunk and extremities with minimal mucosal lesions. Histopathological analysis revealed a subepidermal blister with neutrophilic infiltration with eosinophils in 2 cases. Direct and indirect immunofluorescence studies disclosed IgG and IgA antibasement membrane zone antibodies. In immunoblot analyses of various antigen sources, all cases showed IgG and IgA antibodies to various subunits of laminin-332, in addition to IgG and IgA reactivity with type VII collagen, laminin-γ1, and BP230 and BP180 recombinant proteins. CONCLUSIONS AND RELEVANCE: Our studies revealed that the 3 LAGBD cases showed prominent IgG and IgA reactivity with laminin-332, which was only rarely reported. In addition, all cases showed IgG and IgA reactivity with other multiple antigens, indicating the role of epitope-spreading mechanisms initiated from laminin-332. The significance of IgA antibodies to laminin-332 should be studied in larger cohorts of both LAGBD and linear IgA bullous dermatosis.
Subject(s)
Autoantibodies/blood , Autoantigens/immunology , Cell Adhesion Molecules/immunology , Immunoglobulin A/immunology , Immunoglobulin G/immunology , Linear IgA Bullous Dermatosis/immunology , Aged, 80 and over , Cross Reactions , Female , Fluorescent Antibody Technique, Direct , Fluorescent Antibody Technique, Indirect , Humans , Linear IgA Bullous Dermatosis/pathology , Male , Middle Aged , KalininSubject(s)
Antimetabolites, Antineoplastic/therapeutic use , Bowen's Disease/drug therapy , Human papillomavirus 16 , Mouth Neoplasms/drug therapy , Oxonic Acid/therapeutic use , Papillomavirus Infections/drug therapy , Skin Neoplasms/drug therapy , Tegafur/therapeutic use , Aged, 80 and over , Bowen's Disease/diagnosis , Bowen's Disease/virology , Drug Combinations , Female , Humans , Mouth Neoplasms/diagnosis , Mouth Neoplasms/virology , Papillomavirus Infections/diagnosis , Papillomavirus Infections/virology , Skin Neoplasms/diagnosis , Skin Neoplasms/virologyABSTRACT
Eccrine angiomatous hamartoma (EAH) is a rare, benign cutaneous lesion characterized histologically by a proliferation of eccrine glands and vascular structures-generally capillaries-in the middle and deep dermis. Sudden enlargement of EAH lesions with or without pain has been noted during puberty and pregnancy and has been attributed to hormonal stimulation. We herein describe a case of EAH that became symptomatic in an adolescent girl. A 13-year-old girl presented with pain associated with a sudden enlargement of a previously asymptomatic swelling on her right second toe. She had an 8-year history of an asymptomatic swelling on her right second toe, and the symptoms appeared approximately 1 year after menarche. Physical examination revealed swelling of the plantar surface of her right second toe. The overlying surface was erythematous with a small amount of fine scales. The biopsied tissue showed a nodular proliferation of eccrine glands intimately admixed with numerous small vessels in the deep dermis and subcutaneous fat tissue. Mucin deposition was present in the stroma surrounding the proliferating eccrine coils and ducts and in the upper dermis. A diagnosis of EAH was made. We suggest that hormonal changes during puberty may have played a role in the rapid growth and pain in the present case.
